title: Embryonal rhabdomyosarcoma with metastases confined to the lungs: report from the CWS Study Group.
abstract: BACKGROUND
  Embryonal rhabdomyosarcoma [RME] is the most common pediatric soft tissue sarcoma. Whereas the prognosis of localized rhabdomyosarcoma has improved, it remains poor for metastatic disease.
  
  
  METHODS
  We analyzed RME-patients with isolated pulmonary metastases [PRME] treated in four consecutive CWS-trials. Treatment included multiagent chemotherapy and local treatment of the primary tumor. Therapy of lung metastases after induction chemotherapy depended on response and individual decisions.
  
  
  RESULTS
  Twenty-nine patients <21 years had PRME. Their median age was six years, the median follow-up nine years. Twenty-eight children had their primary tumor located in an unfavorable site and 22 of the primaries were >5 cm. In addition to conventional chemotherapy, seven patients received high-dose treatment and eight patients oral metronomic chemotherapy. The lung metastases were in remission after induction chemotherapy in 22 individuals. 19 patients received no local treatment of metastases; 3 patients had pulmonary metastasectomy and lung radiation was administered to 9 individuals. In total, 24/29 patients achieved a complete remission [CR]. Actuarial 5-year event-free and overall survival for all patients was 37.9 ± 18% and 48.7 ± 18%, respectively; it was 45.8 ± 20% and 58.3 ± 20% for the 24 patients who achieved a CR. Local treatment of metastases had no impact on the failure pattern. Younger age, good response, achievement of CR and maintenance-treatment were favorable prognostic factors in univariate analysis.
  
  
  CONCLUSIONS
  Children with PRME have a fair prognosis. Local treatment of metastases did not improve outcome in our sample. Metronomic treatment may be an attractive option for PREM-patients.
authors.affiliation: Pediatrics 5 (Oncology, Hematology, Immunology), Olgahospital, Klinikum Stuttgart, Germany. tobias.dantonello@olgahospital-stuttgart.de
authors.forename: Tobias M
authors.initials: TM
authors.lastname: Dantonello
authors.forename: Peter
authors.initials: P
authors.lastname: Winkler
authors.forename: Tobias
authors.initials: T
authors.lastname: Boelling
authors.forename: Godehard
authors.initials: G
authors.lastname: Friedel
authors.forename: Irene
authors.initials: I
authors.lastname: Schmid
authors.forename: Adrian C
authors.initials: AC
authors.lastname: Mattke
authors.forename: Gustaf
authors.initials: G
authors.lastname: Ljungman
authors.forename: Stefan S
authors.initials: SS
authors.lastname: Bielack
authors.forename: Thomas
authors.initials: T
authors.lastname: Klingebiel
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007390:International Agencies; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary* / Q000601:surgery; D008207:Lymphatic Metastasis; D008297:Male; D011879:Radiotherapy Dosage; D012074:Remission Induction; D018233:Rhabdomyosarcoma, Embryonal / Q000188:drug therapy / Q000556:secondary* / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome